|
Medullary cystic disease (MCD) is an inherited condition that affects the kidneys. In this condition, multiple small fluid-filled sacs, called cysts, form inside the kidneys.
What is going on in the body?< br>In medullary cystic disease, small cysts form in a part of the kidney called the medulla. Though MCD is inherited, the exact cause is not known. A problem of the tissue deep inside the kidneys is suspected. The end results of MCD include:
- inflammation in the kidney, eventually leading to scarring
- impairment in the ability of the kidneys to secrete and absorb salts
- impaired ability of the kidneys to concentrate and dilute urine
There are different types of MCD. Each usually leads to kidney failure. The age at which kidney failure occurs depends partly on the type of MCD. Abnormalities in other areas of the body, such as vision changes, may also occur with some types of MCD. Most people with MCD either die from the disease or need permanent dialysis or a kidney transplant. Dialysis is a procedure to filter the blood when the kidneys aren't working.
What are the causes and risks of the disease?
Medullary cystic disease is inherited in different ways depending on the type of MCD. A child may need to inherit only one abnormal gene from one parent or one abnormal gene from each parent.
What are the treatments for the disease?
Medullary cystic disease cannot be reversed, so treatment is directed at the complications. Treatment with fluids or salts may be needed to correct dehydration or salt imbalances. Blood transfusions may be needed to treat anemia. The treatment options for kidney failure may include dialysis or kidney transplant. Surgery to remove a kidney may be needed if chronic abdominal pain occurs. Kidney infections are common and are treated with antibiotics. Other treatment will depend on any underlying conditions that result from MCD or kidney failure, such as visual impairments, high blood pressure, or infections.
What are the side effects of the treatments?
All medications and surgeries have possible side effects. For instance, medication for high blood pressure may cause fatigue, allergic reactions, or erectile dysfunction. Antibiotics can cause stomach upset and allergic reactions. Surgery carries a risk of bleeding, infection, and allergic reactions to anesthesia. Dialysis has many side effects, including infection, salt imbalances, and weakness.
What happens after treatment for the disease?
Treatment usually lasts for life, as medullary cystic disease cannot be cured and usually gets worse over time. Once kidney failure occurs, dialysis is usually needed 3 times a week. If a kidney transplant is performed, the person will need lifelong treatment.
How is the disease monitored?
The person's symptoms as well as kidney function tests are used to monitor the degree of kidney failure. Any new or worsening symptoms should be reported to the healthcare provider.
|
